Publications by authors named "Achiya Z Amir"

Aim: This retrospective study aimed to assess the efficacy of prophylactic antibiotics in preventing ascending cholangitis following Kasai portoenterostomy (KPE). Data from 72 patients treated across four tertiary centres in Israel from 2008 to 2018 were analysed.

Methods: Clinical and laboratory data were collected from biliary atresia (BA) diagnosis until liver transplantation (LT) or study completion.

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  • This study aimed to compare the clinical course of acute pancreatitis (AP) in children with inflammatory bowel disease (IBD) versus those without, while also identifying risk factors for AP among IBD patients.
  • The research analyzed data from 68 pediatric patients hospitalized for AP between 2005 and 2019, finding that 13 had a co-diagnosis of IBD, and they experienced milder pancreatitis with no need for invasive treatment.
  • The only significant risk factor for developing AP among IBD patients was the presence of IBD-associated arthritis, while other factors related to IBD and medication showed no significant links to AP development.
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  • The study investigates the effects of oral vancomycin on inflammatory bowel disease (IBD) in patients with primary sclerosing cholangitis (PSC) using data from the Paediatric PSC Consortium.
  • A retrospective cohort of 113 PSC-IBD patients was analyzed, comparing 70 treated with vancomycin to 210 untreated ones, focusing on clinical remission after one year.
  • Results show vancomycin significantly improves odds of both clinical and endoscopic remission, highlighting the need for further randomized controlled trials to confirm these findings and assess safety and dosing.
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  • * A study of Bukharan Jewish patients revealed that this variant is linked to severe forms of the disease, with symptoms often presenting in children around 8.7 years old, including liver issues.
  • * The detected carrier frequency of the variant is approximately 1 in 30, suggesting that screening for this genetic variant should be incorporated into pre-symptomatic and newborn screening programs to prevent serious health complications.
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  • The study explores the use of liraglutide, a GLP-1 analogue, as an off-label therapy for patients with rare genetic causes of early-onset obesity and related complications.
  • Three case studies demonstrated that liraglutide can significantly halt weight gain, improve metabolic parameters, and support overall health in affected individuals despite their complex genetic backgrounds.
  • The findings suggest that liraglutide may have broader benefits beyond weight loss, making it a potentially valuable treatment option for managing obesity-related conditions in genetically predisposed patients.
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Aim: Temporal changes in common pathogens that cause clinical dysentery have been described in Europe. We aimed to describe the distribution of pathogens and their antibiotic resistance in hospitalised Israeli children.

Methods: This study retrospectively studied children hospitalised for clinical dysentery, with or without a positive stool culture, from 1 January 2016 to 31 December 2019.

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  • The study evaluates the effectiveness of routine tissue sampling in children undergoing esophagogastroduodenoscopy and ileocolonoscopy, exploring the agreement between endoscopic and histopathological findings.
  • It analyzed data from 541 endoscopies conducted in 2019, revealing varying sensitivities and specificities across different gastrointestinal regions, with ileocolonoscopy showing higher agreement than esophagogastroduodenoscopy.
  • The findings indicate that while endoscopic results are specific for detecting histological issues, a lack of visible abnormalities does not reliably predict normal histology, supporting the recommendation for routine biopsies in pediatric procedures.
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Celiac disease clinical presentation is constantly changing. We set to determine the prevalence of elevated transaminases in newly diagnosed celiac patients and to evaluate this sub-group of patients for associated clinical and laboratory findings and assess their natural course of disease following therapeutic diet initiation. We conducted a prospective-observational study of all newly diagnosed pediatric celiac patients, between August 2016 and April 2018, in a pediatric gastroenterology clinic.

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  • The study investigates a specific genetic variant in the EMC10 gene, found in multiple individuals with similar neurodevelopmental issues like intellectual disability and developmental delays.
  • Researchers used advanced sequencing techniques to trace this variant across several consanguineous families and confirmed its connection to the observed phenotypes.
  • The findings suggest that a homozygous loss-of-function variant in EMC10 causes a new type of syndromic neurodevelopmental disorder, likely stemming from a hypermutable region of DNA.
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Background And Aims: Many children with primary sclerosing cholangitis (PSC) receive oral vancomycin therapy (OVT) or ursodeoxycholic acid (UDCA). There is a paucity of data on whether these medications improve outcomes.

Approach And Results: We analyzed retrospective data from the Pediatric PSC Consortium.

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Background And Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC.

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Inflammatory bowel disease (IBD), especially when associated with primary sclerosing cholangitis (PSC), is a risk factor for developing colorectal cancer (CRC). We aimed to determine the incidence of CRC in a large cohort of pediatric-onset PSC-ulcerative colitis (UC) patients.

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Background: Natural history models for primary sclerosing cholangitis (PSC) are derived from adult patient data, but have never been validated in children. It is unclear how accurate such models are for children with PSC.

Methods: We utilized the pediatric PSC consortium database to assess the Revised Mayo Clinic, Amsterdam-Oxford, and Boberg models.

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Objective: To investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis.

Study Design: We retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year.

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Objectives: The course and evolution of pediatric acute pancreatitis (AP) is poorly understood. Prognostication models in children perform poorly and lack consensus. We aimed to identify predictors of AP severity, and the risk for AP recurrence.

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Objectives: To describe the prevalence, natural course, outcome, and risk factors of post-transplant de novo allergy and autoimmunity.

Study Design: A cross-sectional, cohort study of all children (<18 years) who underwent a solid-organ transplantation, between 2000 and 2012, in a single transplant center, with a follow-up period of 6 months or more post-transplant and without history of allergy or immune-mediated disorder pretransplant.

Results: A total of 626 eligible patients were screened, and 273 patients (160 males; 59%) met the inclusion criteria; this included 111 liver, 103 heart, 52 kidney, and 7 multivisceral recipients.

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Unlabelled: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death.

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The congenital dyserythropoietic anemias (CDAs) are a group of rare genetic disorders characterized by ineffective erythropoiesis and the development of secondary hemochromatosis. Distal limb anomalies are a well-documented though rare feature of congenital dyserythropoietic anemia type I, that have not been reported so far in other types. We describe a patient with congenital dyserythropoietic anemia type II and four members of a family with clinical features of congenital dyserythropoietic anemia type III with distal limb anomalies.

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Objectives: Surveillance of hepatic nodules for malignant transformation to hepatocellular carcinoma is important in the monitoring of patients with biliary atresia (BA). To date, only 2 published case reports describe the finding of hepatoblastoma (HB) in this setting. The present study aimed to investigate this association of HB and BA, and to assess the utility of alpha-fetoprotein (aFP) as a marker in the diagnosis.

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Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening systemic disease, characterized by overwhelming stimulation of the immune system and categorized as primary or secondary types. Occasionally, acute liver failure (ALF) may dominate the clinical presentation. Given the systemic nature of HLH and risk of recurrence, HLH is considered by many a contraindication to liver transplantation (LT).

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Therapies that promote tolerance in solid organ transplantation will improve patient outcomes by eliminating the need for long-term immunosuppression. To investigate mechanisms of rapamycin-induced tolerance, C3H/HeJ mice were heterotopically transplanted with MHC-mismatched hearts from BALB/cJ mice and were monitored for rejection after a short course of rapamycin treatment. Mice that had received rapamycin developed tolerance with indefinite graft survival, whereas untreated mice all rejected their grafts within 9 days.

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